Clinical and pathological features and surgical treatment of Budd-Chiari syndrome-associated hepatocellular carcinoma.

Background: Budd-Chiari syndrome (BCS) is characterized by liver sinusoidal congestion, ischemic liver cell damage, and liver portal hypertension caused by hepatic venous outflow constriction. The aim of this research was to investigate the clinicopathological features of BCS-associated hepatocellular carcinoma (HCC) and explore its surgical treatment and prognosis.

Methods: Clinical data from 38 patients with BCS-associated HCC who were surgically treated in our hospital from July 1998 to August 2010 were retrospectively analyzed. The clinicopathological features and prognosis of patients with BCSassociated HCC and surgical treatment for BCS-associated HCC were investigated.

Results: Compared to the patients with hepatitis B virus (HBV)-associated HCC, the patients with BCS-associated HCC showed a female predominance, and had significantly higher cirrhosis rate, higher incidence of solitary tumors, lower incidence of infiltrative growth, higher proportion of marginal or exogenous growth, lower rate of portal vein invasion, and higher degree of differentiation. Median survival was longer in patients with BCS-associated HCC (76 months) than in those with HBV associated HCC (38 months). Of 38 patients with BCS-associated HCC, 22 patients who received combined surgery mainly by liver resection plus cavoatrial shunts exhibited hepatic venous outflow constriction relief, while the other 16 patients only underwent liver resection. The combined surgery group had significantly longer survival and lower incidences of post-operative lethal complications (P < 0.05). Multivariate analysis showed that relief of hepatic venous outflow obstruction was a protective factor for survival of patients with BCS-associated HCC, whereas portal vein invasion was a risk factor.

Conclusions: BCS-associated HCC has a more favorable biological behavior and prognosis than HBV-associated HCC. For patients with BCS-associated HCC, tumor resection accompanied with relief of hepatic venous outflow obstruction can reduce the incidence of complications and extend survival.